Eight patients with Larsen's syndrome were investigated and found to have a characteristic pattern of spinal deformity consisting of vertebral anomalies, spondylolysis, and scoliosis. The cervical spine was the most severely involved, and dysraphism as well as hypoplasia represented the most consistent patterns of deformity. Scoliosis was the most common deformity seen in the thoracic spine, whereas dysraphism, scoliosis, and spondylolysis were common in the lumbar spine. Dysraphism again was the most common anomaly in the sacrum. In the young patient, the spine may be difficult to evaluate because major portions of the elements are cartilaginous, and serial roentgenographic evaluations may be required. Early treatment may minimize the severity of the deformities and prevent sequelae.